Hemochromatosis

Hemochromatosis


HEMOCHROMATOSIS

  • Hemochromatosis is a common inherited disorder of iron metabolism leading to deposition of excessive amount of iron in parenchymal cells leading to fibrosis and organ failure.
  • The iron storage pigment in tissues is called as hemosiderin (least in skin).
  • Hemosiderosis is an acquired condition.
  • Liver is the first organ to be affected & maximum deposition of iron by hemochromatosis.
  • Classic triad is- cirrhosis, diabetes mellitus, skin pigmentation.

Conditions of Iron Overload States– classified as

I) Herediatry- hemochromatosis

II) Acquired overload-

a) Iron loading-

  1. Thalassemia major
  2. Sideroblastic anaemia
  3. Chronic haemolytic anaemia

IRON POISONING

  • Ingestion of a number of ferrous sulphate tablets may cause acute iron poisoning.
  • Clinical features : Severe vomiting ,Diarrhea ,GI bleeding ,there may be severe shock, hepatic & renal failure
  • Antidotes:Desferrioxamine is given parenterally (IM or IV), whereas deferiprone and deferasirox are used orally.

Exam Important

  • The iron storage pigment in tissues is called as hemosiderin (least in skin).
  • Hemosiderosis is an acquired condition.
  • Liver is the first organ to be affected & maximum deposition of iron by hemochromatosis.
  • Conditions of Iron Overload States– classified as

I)Herediatry- hemochromatosis

  • Iron loading-
  1. Thalassemia major
  2. Sideroblastic anaemia
  3. Chronic haemolytic anaemia
  • Antidotes for iron poisoning:Desferrioxamine is given parenterally (IM or IV), whereas deferiprone and deferasirox are used orally.
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